Wilms tumor is the most common malignant renal tumor in childhood, but it is rarely associated with a vascular tumor thrombus. We present a case of Wilms tumor with renal vein tumor thrombus treated with only a 3-port retroperitoneal laparoscopic technique in a 10-year-old girl. The patient was alive at the 12-month follow-up examination with adjuvant chemotherapy. To our knowledge, we present the first case of Wilms tumor with renal vein tumor thrombus treated with pure retroperitoneal laparoscopic management. Our technique can be recommended owing to the benefits of the superior recovery profile, a safer surgical route, and cosmetically more acceptable incisions. Crown Copyright (C) 2013 Published by Elsevier Inc.